| Animal models of chronic lung infection with Pseudomonas aeruginosa: useful tools for cystic fibrosis studies. | |
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MedLine Citation:
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PMID: 18782827 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Cystic fibrosis (CF) is caused by a defect in the transmembrane conductance regulator (CFTR) protein that functions as a chloride channel. Dysfunction of the CFTR protein results in salty sweat, pancreatic insufficiency, intestinal obstruction, male infertility and severe pulmonary disease. In most patients with CF life expectancy is limited due to a progressive loss of functional lung tissue. Early in life a persistent neutrophylic inflammation can be demonstrated in the airways. The cause of this inflammation, the role of CFTR and the cause of lung morbidity by different CF-specific bacteria, mostly Pseudomonas aeruginosa, are not well understood. The lack of an appropriate animal model with multi-organ pathology having the characteristics of the human form of CF has hampered our understanding of the pathobiology and chronic lung infections of the disease for many years. This review summarizes the main characteristics of CF and focuses on several available animal models that have been frequently used in CF research. A better understanding of the chronic lung infection caused particularly by P. aeruginosa, the pathophysiology of lung inflammation and the pathogenesis of lung disease necessitates animal models to understand CF, and to develop and improve treatment. |
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Authors:
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I Kukavica-Ibrulj; R C Levesque |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Review Date: 2008-09-09 |
Journal Detail:
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Title: Laboratory animals Volume: 42 ISSN: 0023-6772 ISO Abbreviation: Lab. Anim. Publication Date: 2008 Oct |
Date Detail:
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Created Date: 2008-10-08 Completed Date: 2008-12-18 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0112725 Medline TA: Lab Anim Country: England |
Other Details:
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Languages: eng Pagination: 389-412 Citation Subset: IM |
Affiliation:
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Centre de Recherche sur la Fonction, Structure et Ingénierie des Protéines, Pavillon Charles-Eugène Marchand, Biologie Médicale, Faculté de Médecine, Université Laval, Québec G1K 7P4, Canada. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Cystic Fibrosis / microbiology*, pathology* Disease Models, Animal* Humans Pseudomonas Infections / microbiology*, pathology* Pseudomonas aeruginosa / growth & development* |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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